Helping Children with Pulmonary Hypertension Cope

“He is uncomfortable changing into his gym clothes for fear of showing everyone his central line.”

“She's having a hard time connecting with anyone her own age, maybe because she wants to talk to someone who has gone through the same things she has. Her friends can't relate to her and she can't relate to older people from the support group.”

“We wanted to shut her in a big plastic bubble and keep her safe from anything that could cause her to get sick.”

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What to Expect

When a child is diagnosed with PH, families face a whole new set of circumstances and challenges. As a parent, it’s natural to want to shelter your child from pain. When parenting a child with PH, this isn’t always easy, and between the symptoms, side effects, treatments, and procedures, sometimes it’s just not possible. Some parents say they feel guilty or resentful that they can’t protect their children from the discomfort associated with this disease. It’s also natural to worry about your child’s emotional well-being. Whether your family has been living with PH for a few weeks or several years, it’s normal to have lots of questions about raising a child with a chronic illness, and what you can do to help your child cope.

Many parents find that PH quickly assumes a central role in all aspects of their parenting. Decisions about a sick child’s chores, homework, play dates, and bedtime become more complicated than they might be for a healthy child. Parents have to think about how much responsibility and independence they feel comfortable giving their child, how to stay attentive to their child’s needs, and how to talk about the disease with someone so young.

Parents have the ongoing job of identifying and working through not only their own concerns, but their child’s as well. Some children will speak up as issues present themselves, while others may need gentle encouragement to articulate their feelings and questions. Children also exhibit nonverbal behaviors that can signal confusion, distress or frustration. Pay attention to your child’s moods, facial expressions and body language. Whether or not they’re expressing them, many children have questions, either about about their illness or about the differences they observe between themselves and their siblings or friends. Children may wonder why they can’t run as far as their friends or why they get so much special attention from adults. They might fear pain or assume they became sick because they were “bad.” Your child's questions and concerns will change over time. The next section provides a basic overview of developmental stages to help you address your child's needs at every age.

As time goes on, new challenges will continue to present themselves. Just as you’re finally adjusting to one aspect of parenting a child with PH, an impending doctor’s appointment, a change in your child’s health, or a period of transition (to school, college, or adult care) will bring a flood of fresh questions and concerns. While helping your child understand and cope with all these ups and downs may feel overwhelming, remember that your role as a parent remains constant: to provide the love and support necessary for your child to grow and learn.

Moving Forward

John Wilmot wrote of parenting, “Before I got married I had six theories about bringing up children; now I have six children, and no theories.” Despite all the books and blogs available on the subject, many parents find that all the experts and advice out there can’t compare to the lessons they learn by waking up every day and spending time with their children. Raising a child with PH can be eye-opening, nerve-racking, hectic, physically taxing, emotionally draining, and tremendously rewarding, sometimes all in the same afternoon. By checking in with yourself and your child on a regular basis and trusting your instincts, it's possible to be there for your child through the ups and the downs.

Explaining PH to your Child

Talking about PH with your child can be one of the most challenging tasks parents in the PH community face. As with any sensitive topic, it’s a good idea to plan ahead and anticipate potential questions and stumbling blocks. If you have a co-parent, talk to him or her about how and when to have these conversations, and how much information you feel comfortable sharing when you do. One mother compared explaining PH to her daughter to “having the sex talk.” She went on, “How much to tell is all about age, maturity, and their desire to know.”

The established developmental stages, described here, can help guide these talks, but keep in mind that all children are individuals who process feelings and concepts differently.

Preschoolers are concrete thinkers. They aren’t able to process abstract information, so they may come to the conclusion that something they did caused their PH. They will need reassurance that this is not the case. Preschoolers process their understanding through play, so dolls, books and toys can be helpful tools to illustrate medical scenarios and parts of the body.

Elementary school childrenare beginning to visualize on an abstract level, but they may still believe that PH was caused by something they did. If they are articulating questions they are also imagining possible answers, so honest and clear explanations are important. Simple language such as “your heart works harder” can be helpful.Children may need many invitations to talk before they feel comfortable voicing a concern. Expressing your feelings can encourage children to do the same. Movies, stories and songs can be useful tools in drawing out children who are reluctant to share.

Teenagers have a greater understanding of symptoms, medications and the impact of personal decisions on their health. Still, don't assume an adolescent understands something if you haven't talked about it. At this age, it's ok to ask more leading questions to encourage conversation. Keep in mind that teens have a tendency to turn to the Internet and friends for information. Steer them towards reliable sources to ensure that the information you're providing is reinforced by credible websites and individuals. Teen years are a good time to encourage greater “ownership” of PH and begin the transition to independent adult care.

At every age, try to be gentle, open and honest. If you don't think your child is ready for certain details, that's ok. Some parents find that their children’s questions are the best guide for what to tell and when. One parent shared that she didn’t tell her nine-year-old daughter very much initially except that “she had to take meds to make her heart stronger.” You're the best judge of your child's personality and moods, so you know better than anyone how much information will help your child understand and cope at every stage.

As every parent knows all too well, sometimes children will take you off guard by asking something that you're not prepared to talk about. Around the second grade, some children begin asking about death. Keep in mind that this is normal, even in healthy children. It's in elementary school that children begin to develop a curiosity about what happens when a person dies and their own mortality, particularly if someone in your family has recently passed away. Be honest and open, and give your child the space to voice any fears and questions. Try not to rush the conversation because you're feeling uncomfortable. Children can pick up on anxiety in adults, and evasive responses may only add to their fears. If your family has spiritual beliefs that you want to share with your child, this can be a good time to do that.

Above all, reassure your child that your love is unconditional and, if it comes up, that PH didn’t develop because of anything he or she did wrong. Offer your child an open line of communication. Be on the lookout for signs of confusion or distress, and turn to your PH team, your child’s school counselor, a therapist or clergy member if you or your child need extra support at any point.

Appointments and Hospital Visits

Visits to the doctor’s office or hospital, whether routine or not, can be difficult times for children of all ages. They may be afraid of certain tests and procedures, anxious at the thought of interacting with strangers, put off by the clinical atmosphere, or worried about missing school or social activities.

Children have a tendency to feel anxious about the unknown. If possible, prepare your son or daughter for appointments and hospital visits in advance by describing the appointment or procedure, who will be in the room, what devices will be used, and how much your child will feel during any tests or procedures. How much you tell your child and how far in advance will depend on age and developmental stage. One strategy is to wait the equivalent number of days before an appointment as your child’s age in years (four days for a four-year-old, two weeks for a 14-year-old), but this guideline will vary from child to child. Talk to your PH team for ideas about how to talk to your child about medical tests, as it’s likely they have time-tested strategies for explaining complicated topics to young patients. Some hospitals have pediatric social workers and child life specialists who can help you prepare your child for hospitalizations.

If your child gets a line infection or experiences another unexpected medical emergency, you may not have time to prepare your child for a hospitalization in advance. Unplanned visits to the hospital can be frightening for both parent and child. You can reduce your own stress in these situations by having an emergency plan ready and following it when the time comes. Pack a bag of essentials to keep near your front door. Include a change of clothing for each family member, books and magazines, comfort objects for your child, nonperishable snacks, $20 in cash for parking, and your child's medication list. Keep a supply of pre-made meals in your freezer and ask two to three neighbors or family members to serve as "on-call babysitters" for your other children or pets if you need to leave the house in a hurry. Children have a tendency to take cues from adults in stressful situations. If you're able to manage your own tension and project a calm demeanor, your child will be more likely to follow suit. On the trip to the hospital, pay close attention to your child, responding to any questions with simple and reassuring language. If possible, use distraction techniques like singing or car games to take you child's mind off any pain or anxiety.

Once at the hospital, stay attentive to your child’s body language for signs of discomfort. Keep in mind that it can be difficult for young people to interact with adults they don’t know. Children are sometimes embarrassed to ask nurses for help. Explain that nurses spend time with other kids with conditions like PH every day. Stay at your child’s side whenever possible, and work with the doctor and nurse to explain what’s happening to put your child at ease. You can actively participate in your child’s care during a hospitalization by staying overnight or helping in tasks such as washing. Answering honestly to questions such as “will this hurt?” helps build trust between parent and child and removes the fear of the unknown.

Many parents also try to ease the anxiety their children feel around appointments and procedures by concentrating on the positive. You might focus conversation around how great it will be to feel better after surgery, or all the visitors and games there are to look forward to during a hospital stay. Hannah, now in her 20s, fondly remembers her parents’ strategy for relieving her hospital-related stress. After blood draws, IVs, PICC lines, and heart caths, she would always receive a small gift. She told us, “I wasn’t spoiled … I never got money for good grades, but that was something simple that my parents did to keep my mind off the pain. To this day I often go get an ice cream after a less-than-fun doctor’s visit.”

At Home

With so much already on a young patient’s plate, it might be tempting to avoid piling on additional responsibilities. But be wary of protecting your child too much. To help a chronically ill child feel as much like a normal kid as possible, professionals recommend that parents set limits and encourage responsibility, just as you would with a child who didn’t have PH.

One of the easiest places to establish this sense of normalcy is at home. Talk about your expectations for good behavior and help around the house. One mom, Christine, created a list of chores that she knew her daughter could complete successfully, PH and all. Make sure there are consequences if your expectations aren’t met and rewards for jobs well done. Get in a routine and stick with it. Having a predictable home life, with fairly consistent chores, meal times, bedtimes and activities, can counteract the stress and unpredictability of the ups and downs of PH. A well-thought-out routine can also ensure that you make every effort to give every family member, including healthy children and your spouse, the attention they deserve.

As time goes on, you can also encourage your child to take on new responsibilities regarding his or her health and medication management. While parents sometimes feel the need to control all aspects of their children’s health, offering small choices in a predictable environment can ease the inevitable transition to adult care. One parent told us, “We allowed our daughter to choose her own bedtime during the summer as long as she was responsible in taking her oral medications on a daily basis.” When the daughter made choices that made her feel worse, she learned to avoid them in the future, and thus slowly developed her own action plan for tending to her health. You can help your child identify activities or behaviors to avoid by verbalizing the circumstances and symptoms when he or she doesn’t feel well. You might also encourage your child to mix his or her own medications or fill his or her own pill box occasionally. As children get older, their confidence will grow and they will feel empowered to make more and better choices.

Remember that life with a chronic disease can be extremely stressful for a child, so it’s a good idea to keep the home environment as stress-free as possible. While there will be hard times and difficult conversations ahead, try not to argue with your partner about PH or medical bills within earshot of your child. Instead, create a safe and supportive atmosphere by showing your child that you, your partner and the PH medical team are all working together to give the young patient the care he or she deserves.

School, Friends and Fitting In

Planning for school can be particularly stressful to parents of young PHers because it’s a place where they have less hands-on control of their child’s care than they do at home. One parent told us that with all the dangers and unknowns she feared, she debated if she should let her daughter attend school at all. She worried, “What if she fell? Her line could get pulled, the kids could pick on her, and she may get too tired!” Yet the knowledge, confidence and relationships children build in school (or, in the case of home-schooled kids, extracurricular activities) are important building blocks in their growth and development. Work through your own fears in advance so you can help your child cope with his or her feelings as they arise.

Speaking to the school in advance in order to prepare teachers for any special needs your child has can help ease some of these anxieties. PHA has a School Resource Handbook you can use to work with school personnel to ensure that your child’s classroom environment is as safe and supportive as possible (see Additional Resources). Eventually your child can begin to participate in the process of educating teachers, counselors, and coaches about PH. One high school student liked to take the lead in the meetings her parents convened of all her teachers at the beginning of every year. She said, “I felt in charge of my PH and my situation at school.”

Often parents worry that children with PH will become alienated from their peers because they feel so different. Once children are settled in a new routine, however, most of the stories we hear are positive ones. One mother shared, “Katy has always had a couple of good friends. Her classmates have always protected her and cared about her.” Ask the teacher to treat your child like any other student, and students will usually follow the teacher’s example. And the more you can do to help demystify your child’s illness, the better. Children are naturally curious, so you might consider visiting the classroom to explain the basics of PH, oxygen or an infusion pump to your child’s classmates.

One mother was relieved to confide, “Sarah made a ton of new friends and they all wanted to learn about her pump and backpack.” A network of informed friends can give peace of mind to both children and their parents. One father said, “Iain has been able to develop very strong friendships with kids who have been with him since diagnosis. This network of friends and their parents helps us to provide a secondary level of protection for him.”

The older they get, the more children with PH might dwell on the negative aspects of their disease. Depending on the severity of their symptoms or the nature of their medications, they may be unable to participate in some physical activities. One parent recommended creating “a list of what PHers can do, like watch a movie or play a board game or video games.” While there’s a chance you’ll have to say no to some activities, there may be alternatives that are equally appealing to your child. Remember, for every activity that children with PH can’t do, there’s one they can do. One mom said of her nine-year-old with PH, "She's a pretty typical kid. She can't run well or keep up with the other kids, of course. But she's very bright and typically sassy. She has big plans for her future and to live a long, happy life."

During the self-conscious teen years it can hurt a lot to feel different from peers. Some young patients have difficulty accepting ownership of the disease as adulthood approaches. Open lines of communication are vital during this time to discuss new responsibilities, drinking, sexual activity and drug use. Periods of denial or some non-compliance are also normal in teens. Talk openly with your child if this occurs. If it continues, speak to his or her PH doctor for advice.

Many kids get through tough times by making friends with other young people living with PH. Some parents coordinate visits to the PH doctor to coincide with the visits of other children with PH of similar ages. You can use PHA’s email group, message board or support group calls to meet other parents in your area. Attending PHA’s biennial International PH Conference is another great way for children to meet other kids who understand what they’re going through. Online, kids can visit PHA’s website to read interviews with other kids and get advice on school, sports and more. Teens (ages 13 to 18) can visit PHA’s secure social network, PHA Teens, to meet other teenagers living with the illness. In the summer, some parents arrange for their kids and teens to attend camps that cater specifically to children with heart disorders and other chronic diseases. See Additional Resources to learn more about all of these options.

Quick Tips

Before discussing PH with your child, acknowledge and work through your own issues to prevent projecting your own fears or concerns onto the conversation.

Ask questions such as “How are you handling things?” or “How can I make things easier for you?” to gauge how much your child understands and how he or she is processing and coping with the situation.

If your child makes a mistake that negatively affects his or her health, try to ask thought-provoking questions (such as, “How do you think your heart is doing if you delay taking your medicine?”) rather than getting upset.

If you’re worried about giving your child more independence, do everything you can to educate teachers, friends, and others in your child’s life about PH.

Consider giving your child a cell phone to call you or medical contacts in an emergency.

Side effects such as facial flushing can be embarrassing, especially during the teen years. Encourage your teen to discuss these issues openly with his or her doctor.

Work with your child’s school to accommodate his or her PH. If your child tires easily, a shortened school day might be arranged. Gym class could be made easier by arranging in advance for your child to change alone if he or she is self-conscious (of supplementary oxygen or a central line, for example) or signaling to the teacher when he or she is tired.

If your child has trouble participating in sports and physical play, be proactive about scheduling in plenty of sedentary activities and hobbies (like photography, strategy games and writing) that appeal to your child's interests.

PHA is thankful to Michele Calderbank, Pulmonary Hypertension Coordinator at Children’s Hospital, Colorado, for her input and medical review, and to the parents and children who shared their experiences to make this resource possible.

The information provided on the PHA website is provided for general information only. It is not intended as legal, medical or other professional advice, and should not be relied upon as a substitute for consultations with qualified professionals who are familiar with your individual needs.